We report a 29 years old male with a non obstructive hypertrophic cardiomyopathy that survived two episodes of cardiac arrest and with a familiar history of the disease and sudden death. He had an implant of an automatic implantable cardioverter defibrillator by a left anterior thoracotomy with intraoperative electrophysiology. The postoperative outcome was uneventful. After one year of follow up, the patient is in good functional capacity and the implanted device has not performed defibrillations.